Introduction
A 78-year-old male patient presented to our hospital with
a slowly enlarging, painless right inguinal mass that had been
present for 15 years. On physical examination, a non-tender right
scrotal mass was palpable. Computed tomography revealed a
12-cm encapsulated cystic lesion containing a calculus (Figure
1). Blood tests showed a mildly elevated C-reactive protein
(CRP) level of 0.74 mg/dL (reference range: < 0.14 mg/dL).
Based on these findings, a diagnosis of right scrotal hydrocele
was made, and the patient underwent right orchiectomy with
hydrocelectomy. Intraoperative findings indicated that the mass
was located in the scrotum and extended toward the external
inguinal ring. Orchiectomy was selected because the hydrocele
was firmly adherent to the testis, with chronic inflammation
and fibrosis, making safe separation technically unfeasible.
The resected specimen contained turbid fluid, a calculus, and a
markedly thickened, inflamed, and partially necrotic cyst wall
(Figure
2). Histopathological examination revealed diffuse
thickening of the tunica vaginalis with dense lymphoplasmacytic
infiltration and characteristic storiform fibrosis (Figure
3A). The
testis itself was markedly atrophic, and the seminiferous tubules
showed diffuse atrophy with thickened basement membranes and hyalinization. The hydrocele was not located within the testicular
parenchyma. Immunohistochemical staining demonstrated
27-48 Immunoglobulin G4 (IgG4)-positive plasma cells per
high-power field (HPF) and an IgG4-positive/IgG-positive
plasma cell ratio of 25-55% (Figure 3B). Given the patient's
long-standing, slowly enlarging inguinal mass without prior
hospital visits, and the marked chronic inflammatory changes
observed intraoperatively and pathologically, the exact origin
and progression from the inguinal area cannot be determined.
Figure 1. Computed tomography image. Computed
tomography scan showing a 12-cm encapsulated cystic
lesion with a calcified nodule in the right inguinal region
Figure 2. Gross appearance of the resected specimen showing
a thickened, inflamed, and focally necrotic cyst wall
Figure 3. A- Histopathological examination revealing
lymphoplasmacytic infiltration and storiform fibrosis (H&E
×100), B- Immunohistochemical stain highlighting numerous
IgG4 positive plasma cells (×400)
Serum IgG4 was within normal limits, and no involvement
of other organs was observed. According to the 2019
American College of Rheumatology/European League Against
Rheumatism (ACR/EULAR) classification criteria for IgG4-
related disease [1], a diagnosis of IgG4-related disease (IgG4-
RD) was established. The postoperative course was uneventful,
and the patient remains well on follow-up.
IgG4-RD is a recently recognized, immune-mediated
fibroinflammatory disorder characterized by tissue infiltration
with IgG4-positive plasma cells, lymphoplasmacytic
inflammation, storiform fibrosis, and frequently obliterative
phlebitis [2]. It can involve multiple organs, most commonly the pancreas, salivary glands, and lacrimal glands, resulting in
clinical entities such as autoimmune pancreatitis, sialadenitis,
and dacryoadenitis. Corticosteroid therapy is considered the
first-line treatment, although no randomized controlled trials
have been conducted to date. In the present case, systemic
therapy was not initiated because serum IgG4 was normal and
there was no evidence of extra-scrotal disease.
Reports of IgG4-RD involving the male reproductive
system are rare, with only a few testicular cases described in
the literature. To our knowledge, presentation as chronic scrotal
hydrocele represents an exceptionally uncommon manifestation.
This case underscores the importance of considering IgG4-RD
in the differential diagnosis of long-standing scrotal masses,
particularly when histological features are suggestive. Increased
awareness of this entity may facilitate accurate diagnosis,
appropriate management, and a deeper understanding of its
diverse clinical spectrum.
Ethics Committee Approval: N/A
Informed Consent: An informed consent was obtained from
the patient.
Publication: The results of the study were not published in full
or in part in form of abstracts.
Peer-review: Externally peer-reviewed.
Conflict of Interest: The authors declare that they have no
conflicts of interest.
Financial Disclosure: The authors declare that this study
received no financial support.
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